WBR0621

Author	[[PageAuthor::Mugilan Poongkunran M.B.B.S [1]]]
Exam Type	ExamType::USMLE Step 3
Main Category	MainCategory::Primary Care Office
Sub Category	SubCategory::Gastrointestinal
Prompt	[[Prompt::A 43 year old male, comes to the office with his wife complainting of severe fatigue and lethargy over the past 1 month. On further review of history, he says he had intermittent fatigue, lethargy, nausea, abdominal pain and joint pains for several years for which he never consulted a doctor. His wife says he has loss of libido and erectile dysfunction for the past few years. Previous they had normal sexual life. His past history is otherwise insignificant and his family history is unremarkable. He is a vegetarian and does not smoke and drink. On examinations, his temperature is 36.7 C, blood pressure is 130/70 mmHg, pulse is 72/min and respiration's are 16/min. Abdominal examination reveals mild abdominal pain on the right upper quadrant and profound hepatomegaly. His skin has golden blue hue. The patient is admitted and treated suppourtively. His serum biochemistry results shows Na:135 mEq/L, K: 3.5 mEq/L, Cl:104mEq/L, Bi: 24 mEq/L, BUN: 30 mg/dl, glucose:226 mg/dl, Mg:1mg/dl and Ca: 8mg/dl. His tranferrin saturation is 72%, serum ferritn is 500 ng/ml and serum iron levels are 300 µg/dl. Liver function tests shows very highly elevated bilirubin, AST and ALT levels. Liver biopsy done shows cirrhosis and his renal function tests are highly abnormal which warrants an immediate dialysis. You explain the patients disease to his wife and discuss about his prognosis. Which of the following may pose an increased risk of infection in this patient?]]
Answer A	AnswerA::Vibrio vulnificus
Answer A Explanation	[[AnswerAExp::Incorrect: Septicemia and/or necrotizing fasciitis from Vibrio vulnificus is more common in patients with hemochromatosis who ingest uncooked seafood. The patient being a vegetarian make him unlikely to get this infection.]]
Answer B	AnswerB::Yersenia enterocolitis
Answer B Explanation	[[AnswerBExp::Incorrect: Yersinia enterocolitica is another organism which may pose an increased risk of infection in iron overload states. However it is uncommon than listeria infection in patient undergoing dialysis for renal failure.]]
Answer C	AnswerC::Listeria monocytogenes
Answer C Explanation	[[AnswerCExp::Correct: Hemochromatosis with cirrhosis and transfusional iron overload in dialysis patients both appear to be risk factors for infection with listeria.]]
Answer D	AnswerD::Staphylococcus aureus
Answer D Explanation	[[AnswerDExp::Incorrect: Hemochromatosis and dialysis does not have any relation to staphylococci infection.]]
Answer E	AnswerE::Vibrio parahemolyticus
Answer E Explanation	[[AnswerEExp::Incorrect: Vibrio parahemolyticus does not have any relation to hemochromatosis.]]
Right Answer	RightAnswer::C
Explanation	[[Explanation::Hemochromatosis is a hereditary disease characterized by improper dietary iron metabolism (making it an iron overload disorder), which causes the accumulation of iron in a number of body tissues. C282 Y mutations in HFE gene cause excessive amounts of iron to be absorbed. Iron accumulation can eventually cause end organ damage, most importantly in the pancreas manifesting as diabetes and liver failure. Other manifestations are impotence; loss of libido, osteoporosis, amenorrhea, heart failure and hypothyroidism. Hemochromatosis can increase the risk of infections with specific types of bacteria. Accumulation of iron in immune cells interferes with their ability to fight off certain bacteria, and other bacteria grow well in an iron-rich environment making them prone to yersinia enterocolitica and vibrio vulnificus. Hemochromatosis with cirrhosis and transfusional iron overload in dialysis patients both appear to be risk factors for infection with listeria. Educational Objective: References: ]]
Approved	Approved::Yes
Keyword	WBRKeyword::Hemochromatosis
Linked Question	Linked::
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